A Patient with MSUD: Acute Management with Sodium PhenylacetateSodium Benzoate and Sodium Phenylbutyrate
Joint Authors
Köse, Melis
Canda, Ebru
Kagnici, Mehtap
Uçar, Sema Kalkan
Çoker, Mahmut
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-05-15
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
In treatment of metabolic imbalances caused by maple syrup urine disease (MSUD), peritoneal dialysis, and hemofiltration, pharmacological treatments for elimination of toxic metabolites can be used in addition to basic dietary modifications.
Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate (NaPB) in urea-cycle disorder cases has been associated with a reduction in branched-chain amino acid (BCAA) concentrations when the patients are on adequate dietary protein intake.
Moreover, NaPB in treatment of MSUD patients is also associated with reduction of BCAA levels in a limited number of cases.
However, there are not enough studies in the literature about application and efficacy of this treatment.
Our case report sets an example of an alternative treatment’s efficacy when extracorporeal procedures are not available due to technical difficulties during attack period of the disease.
American Psychological Association (APA)
Köse, Melis& Canda, Ebru& Kagnici, Mehtap& Uçar, Sema Kalkan& Çoker, Mahmut. 2017. A Patient with MSUD: Acute Management with Sodium PhenylacetateSodium Benzoate and Sodium Phenylbutyrate. Case Reports in Pediatrics،Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1149191
Modern Language Association (MLA)
Köse, Melis…[et al.]. A Patient with MSUD: Acute Management with Sodium PhenylacetateSodium Benzoate and Sodium Phenylbutyrate. Case Reports in Pediatrics No. 2017 (2017), pp.1-4.
https://search.emarefa.net/detail/BIM-1149191
American Medical Association (AMA)
Köse, Melis& Canda, Ebru& Kagnici, Mehtap& Uçar, Sema Kalkan& Çoker, Mahmut. A Patient with MSUD: Acute Management with Sodium PhenylacetateSodium Benzoate and Sodium Phenylbutyrate. Case Reports in Pediatrics. 2017. Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1149191
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1149191